Blanched fingers

73-year-old man came in for a routine skin examination, and several of his fingertips felt cool and appeared “blanched” (Figure). The changes, which had been present for 8 years, were most prevalent in colder weather and were asymptomatic.

Figure.

Raynaud's phenomenon with vasoconstriction: (a) ventral, (b) dorsal.

What is the diagnosis?

DIAGNOSIS: Raynaud's disease.

DISCUSSION

Most commonly, clinical examination reveals whiteness or blanching of distal fingertips due to vasoconstriction, as well as edema due to vasodilation and reactive hyperemia, suggestive of Raynaud's. While the fingers are most commonly affected, the toes, nose, tongue, and ear lobes may be involved. The condition is termed Raynaud's disease when the cause is unknown and Raynaud's phenomenon when it has an associated disorder (1). Table 1 outlines the differences between the two entities. Severe cases of Raynaud's disease can bring tightening of the skin, small painful ulcer formation on the fingertips, and platelet clot formation due to tunica intima thickening in distal arterioles (2).

Table 1.

Characteristics of primary vs secondary Raynaud's^∗

Primary (Raynaud's disease)	Secondary (Raynaud's phenomenon)
Younger age at onset	Older age at onset (usually >30 years)
Symmetric attacks	Symmetric or asymmetric attacks
Milder symptoms (absence of tissue necrosis, ulceration, or gangrene)	Often, intense, painful episodes; may be associated with ischemic lesions
Normal nailfold capillaries	Microvascular disease event on microscopy of nailfold capillaries
Absence of symptoms associated with secondary disease state	Presence of symptoms of associated secondary disease (often a connective tissue disorder)
Negative test for autoimmune disease (e.g., ESR, ANA, RF, specific autoantibodies)	Positive test for autoimmune disease
History of migraine headaches and/or Prinzmetal's (variant) angina	History of environmental or occupational predisposing factor

∗From Wigley, 2002 (1).

ESR indicates erythrocyte sedimentation rate; ANA, antinuclear antibodies; RF, rheumatoid factor.

Some authors have reported that Raynaud's disease has a clinical association with pulmonary hypertension, variant angina, and migraine headaches (3). Others have reported that Raynaud's phenomenon may be associated with systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis, and polymyositis (4). Several disorders have been known to cause Raynaud's phenomenon (Table 2). Systemic sclerosis is unlikely if abnormal nailfold capillaries are not present. An associated autoimmune disease can be evaluated by measuring erythrocyte sedimentation rate, antinuclear antibodies, rheumatoid factor, and disease-specific autoantibodies, as well as by performing a complete blood count. Occult malignancies may be present, especially with patients presenting with bone pain. Hyperthyroidism can be ruled out by testing thyroid function (measuring thyroid-stimulating hormone, T4, and T3). While Raynaud's disease can develop into Raynaud's phenomenon, it is unlikely to do so if present for more than 2 years (1, 3).

Table 2.

Causes of Raynaud's phenomenon^∗

• Anorexia nervosa

• Autoimmune diseases

• Cancer

• Connective tissue disease

• Hematologic causes (cryoglobulinemia, cold agglutinins, macroglobulins, polycythemia)

• Hypothyroidism

• Intraarterial injections

• Neoplasms

• Neurologic disorders

• Obstructive arterial disease

• Predisposing drugs (beta-blockers, ergot alkaloids, sympathomimetics, clonidine, narcotics, cocaine, methysergide, some chemotherapeutic agents, estrogen, and cyclosporine)

• Systemic sclerosis

• Trauma (vibration/electric shock/frostbite/meat cutters)

• Vasculitis and hepatitis B antigenemia

• Vinyl chloride exposure

∗From Shinn, 2008 (3).

Occupations that involve continued exposure to cold, typing, use of vibrating equipment, or piano playing can cause Raynaud's disease. In addition, several drugs may predispose patients to Raynaud's disease: beta-blockers, ergot alkaloids, sympathomimetics, clonidine, narcotics, cocaine, methysergide, some chemotherapeutic agents (e.g., bleomycin, vinblastine, cisplatin), estrogen, and cyclosporine. Therefore, patients' symptoms may subside with a change to a low-estrogen or progesterone-only oral contraceptive. Lastly, smoking can increase the symptoms of Raynaud's disease.

Overactive sympathetic reflex vasoconstriction, facilitated by anything that triggers the sympathetic system or leads to catecholamine release (e.g., cold temperatures), could possibly be the cause of Raynaud's (2). The condition may have genetic or hormonal components (3, 5).

Raynaud's is diagnosed largely on clinical examination. No pathognomonic laboratory test can reliably confirm the diagnosis. Clinically, the presence of distorted capillaries in nail folds suggests an underlying connective tissue disease. Raynaud's phenomenon, often out of proportion to skin findings, may be seen in scleroderma and other connective tissue diseases (e.g., systemic lupus erythematosus, dermatomyositis and polymyositis, mixed connective tissue disease, rheumatoid arthritis, Sjögren's syndrome). In 70% of scleroderma cases, Raynaud's phenomenon is the first manifestation. Rapid diagnosis is critical with this disease because it can lead to gangrene and/or need for amputation. A variety of factors hallmark Raynaud's transition to the more severe connective tissue disease: finger swelling, the initial presence of antinuclear antibodies, older age of onset of symptoms, and being female (6).

Pharmacologic treatments are summarized in Table 2. Numerous nonpharmacologic treatments also exist. For example, discontinuing smoking can alleviate Raynaud's disease symptoms since smoking has been shown to worsen the intensity and frequency of attacks because it decreases the delivery of oxygen to peripheral tissue (5, 7). Also, the discontinuation of predisposing drugs can decrease symptoms. Certain emotionally hypersensitive patients may benefit from relaxation therapies. Physicians should also treat any underlying conditions, such as scleroderma, in those with Raynaud's phenomenon (3).

Table 3.

Recommended agents for the treatment of Raynaud's^∗

∗From Wigley, 2002 (1).

SR indicates sustained release; ARB, angiotensin II receptor blocker; ACE, angiotensin-converting enzyme; SSRI, selective serotonin reuptake inhibitor; PDE, phosphodiesterase; bid, twice a day; tid, three times a day.